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MALTA CONGENITAL ANOMALIES REGISTRY

Annual Congenital Anomalies Report 2001

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Contents: ACKNOWLEDGEMENTS: 5 INTRODUCTION TO THE REGISTRY INTERNATIONAL RELATIONSHIPS 7 THE STUDY POPULATION 8 ASCERTAINMENT METHODS 9 DEFINITIONS AND EXCLUSIONS 11 CASE INFORMATION 11 CODING OF CONGENITAL ANOMALIES 12 CALCULATION OF PREVALENCE RATES AND STATISTICAL METHODS 12 REPORT - 2001 INFANTS / FETUSES REGISTERED 15 FIRST SOURCE OF INFORMATION 15 ISOLATED VS MULTIPLE ANOMALIES 16 TIME OF FIRST DIAGNOSIS 16 INFANTS BY GESTATIONAL AGE 17 INFANTS BY BIRTH WEIGHT 17 MATERNAL AGE DISTRIBUTION 18 GEOGRAPHICAL DISTRIBUTION 19 DISTRIBUTION BY MONTH OF BIRTH 20 ANALYSIS OF INFANTS/FETUSES BY ANOMALY SYSTEM INVOLVED 21 TABLES TABLE A PREVALENCE OF ANOMALIES PER 10,000 BIRTHS FOR 2001 24 TABLE B TIME TRENDS CASES AND PREVALENCE PER 10,000 BIRTHS FOR SELECTED ANOMALY GROUPS 1993-2001 27 TABLE C COMPARISON OF PREVALENCE RATES OF SELECTED ANOMALY GROUPS WITH OTHER EUROPEAN COUNTRIES WITHIN EUROCAT 39 ANNEX 1 LOCAL REPORT FORM 41 ANNEX 2 LIST OF EXCLUSIONS 42 ANNEX 3 REGISTRY REPORT FORM 43 ANNEX 4 LIST OF ANOMALY SUBGROUPS 44

Compiled by: Dr. Miriam Gatt

Department of Health Information

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Malta Congenital Anomalies Registry (MCAR)

A branch of the Department of Health Information Address: 95, G'Mangia Hill G'Mangia, MSD 08 Phone: (+356) 21251516, 21242454 Fax: (+356) 21 235910

Internet: http//www.health.gov.mt/ministry/dhi/mcar.htm Contact person: Dr. Miriam Gatt (miriam.gatt@gov.mt)

ACKNOWLEDGEMENTS: The data in this report comes from several sources and its compilation would not have been possible without the work and co-operation of all departments and sections involved and the staff at the Department of Health Information. Acknowledgements go to all doctors and paramedics who notify cases of congenital anomalies, to St. Lukes and Gozo General Hospitals and the various sections which provide access to their records namely the Maternity and Paediatric wards, Cardiac Lab, Genetics Clinic and the St. Lukes Hospital Activity Analysis Database. I am also grateful for the information supplied by the National Obstetric Information Systems Database, the National Mortality Register and the National Cancer Register. Thanks also go to the maternity sections of the private hospitals: St. James Capua, St. James and St. Philips Hospital which report any baby born with congenital anomaly. Special thanks go to the staff of the Malta Congenital Anomalies Registry, namely Ms. Vivienne Parnis as well as the other staff working on national registers: Ms. Josephine Farrugia, Ms. Rita Micallef and Ms. Connie Scicluna. Thanks are also due to Dr. Joe Pace for software and technical support and Dr. Renzo Pace Asciak, Consultant, Department of Health Information.

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INTRODUCTION MALTA CONGENITAL ANOMALIES REGISTER

Congenital Anomalies include structural defects (congenital malformations, deformations, disruptions and dysplasias), chromosomal abnormalities, inborn errors of metabolism and hereditary diseases. They are a major cause of infant mortality, childhood morbidity and long-term disability. They are also a major cause of embryonic and fetal death and are among the leading causes of years of potential life lost. Congenital anomalies carry a high burden to affected individuals, their families and the community in terms of quality of life, participation in the community and need for services. They are therefore of significant Public Health importance. A register of congenital anomalies was first started in Malta in 1985 with data being collected from St. Lukes Hospital (SLH) as part of a research project funded by the University of Malta. In 1997, the running of the register was assumed by the Department of Health Information and a computerised database was developed with computerised data being backdated to 1993. The register presently collects data from all hospitals on the Maltese Islands and utilises multiple sources of information including information from maternity wards at SLH, Doctors notifications, National Obstetric Information Systems database, Cardiac Lab. records, Hospital Activity Analysis database, information from the Genetics Clinic and others. The aims of the Malta register are: to collect data about all fetal deaths and infants with a diagnosis of congenital anomalies on

the islands of Malta and Gozo; to detect any changes in occurrence of congenital anomalies; to keep a register of all cases of congenital anomalies diagnosed until one year of age; to provide data which may be required for epidemiological studies both locally and

internationally; to issue regular reports and provide physicians and the general public with information they

may need, always respecting strict confidentiality. The Malta Congenital Anomalies Registry has a regularly updated web site: http://health.gov.mt/ministry/dhi/mcar.htm. This site contains the most recent data and summary statistics. All data collected by the Registry is processed in accordance with the Data Protection Act, 2001.

INTERNATIONAL RELATIONSHIPS European Registration of Congenital Anomalies (EUROCAT) The Malta Congenital Anomalies Register has been a full member of EUROCAT (http://www.lshtm.ac.uk/php/eeu/eurocat/eurocat.htm) since 1986, regularly transmitting anonymised case-based data to this network. EUROCAT is a European network of population-based registries for the epidemiologic surveillance of congenital anomalies. It was started in 1979 and now more than 900,000 births per year in Europe are surveyed by 36 registries in 17 European countries. EUROCAT Central Registry is currently located at the University of Ulster, N. Ireland and it houses a standardised central database on more than 160,000 cases of congenital anomaly among livebirths, stillbirths and terminations of pregnancy. This database is updated every year.

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The objectives of EUROCAT are: to provide essential epidemiologic information on congenital anomalies in Europe - to facilitate the early warning of new teratogenic exposures, - to evaluate the effectiveness of primary prevention, - to assess the impact of developments in prenatal screening, - to act as an information and resource center for the population, health professionals and

managers regarding clusters or exposures or risk factors of concern, - to provide a ready collaborative network and infrastructure for research related to the causes

and prevention of congenital anomalies and the treatment and care of affected children and - to act as a catalyst for the setting up of registries throughout Europe collecting comparable,

standardised data. As congenital anomalies have a relatively low prevalence and good quality exhaustive data is expensive and difficult to collect, a standard European system allows countries using data from regional registries to pool their data for studies and to exploit their differences by comparing them. European collaboration also allows for sharing of expertise and enables a joint approach to European public health questions. International Clearinghouse of Birth Defects Monitoring Systems (ICBDMS) The Malta Congenital Anomalies Register was accepted as a member of the ICBDMS in 2000, regularly transmitting aggregate data to the Clearinghouse Centre currently located in Rome. This data has been included in the organisations Annual Reports and World Atlas of Birth Defects. The ICBDMS is a non-governmental organisation in official relations with the World Health Organization representing 39 malformation monitoring programs worldwide. Member programs are actively engaged in the systematic collection and analysis of the data for the comprehensive monitoring of congenital malformations. The organization was established in 1974, at a meeting in Helsinki, Finland, where representatives of malformation monitoring systems in ten countries were present. The mission of the ICBDMS is to help local registries of congenital malformations to identify and to prevent birth defects and to serve as an early warning system to avoid the spread of an epidemic of congenital malformations. To accomplish this the ICBDMS has three main objectives:

exchange of routine information in the prevalence of congenital malformations collaborative epidemiologic research expert consultation and assistance for existing monitoring systems to investigate

outbreaks and for helping new monitoring systems get established

THE STUDY POPULATION The Malta Congenital Anomalies Register is population based and covers all births on the islands of Malta and Gozo. All infants/fetuses who are diagnosed or suspected of having a congenital anomaly until one year of age are included. The small size and population of the islands (Area: 316 km2, Population: 385,077 in 2001); the geographically well defined boundaries, absence of significant ethnic minority groups and illegality of termination of pregnancy make the islands ideal for epidemiological studies.

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This report gives a detailed breakdown of all births diagnosed and registered with congenital anomaly in 2001. Denominator data of total livebirths and stillbirths have been taken from the 2001 Demographic Review of the Maltese Islands.

ASCERTAINMENT METHODS To ensure as complete an ascertainment as possible, the Malta Congenital Anomalies Register makes use of active case finding and multiple sources of information. Cases of congenital anomalies diagnosed in the first few days of life in babies born at St. Lukes Hospital are recorded by staff